Conditions 2 Flashcards
What are the characteristics of febrile seizures? (are they harmful?)
- Generalised tonic-clonic seizures, dont last long.
- Period of postictal drowsiness
- Periods of cyanosis/apnoea.
Majority are harmless! 30-40% will develop further febrile seizures.
What are atypical febrile seizures?
> 15mins, or >2 within 24hrs, or a focal seizure.
^risk of epilepsy
Management of a febrile seizure?
TIME it, + prevent child from hurting themselves (don’t restrain)
- > 5mins = Buccal midazolam
- Another 10mins= IV Lorazepam
- > 30mins= treat as status epilepticus
What are the 2 types of Breath Holding Attacks in toddlers?
- Cyanotic spells (breath holding episode)
2. Pallid spells (reflex anoxic seizure)
What is the difference of a breath-holding episode + a reflex anoxic seizure?
- Breath-holding: precipitated by anger/crying > holds breath > goes blue > then limp, rapid recovery (absence of postictal phase)
- Reflex anoxic: precipitated by pain/minor injury > triggers vagal reflex + stops breathing > goes pale > brief seizure sometimes > rapid recovery (absence of postictal phase)
What are generalised epileptic seizures?
Discharge arises from both hemispheres.
- Tonic-clonic (grand mal)
- Absence (petit mal)
- Myoclonic
1) Tonic clonic seizures?
2) Absence seizures?
3) Myoclonic seizures?
1) Tonic: LoC, limbs extend, back arches + breathing stops. Teeth clenched. Clonic: Intermittent jerking, irregular reathing + micturition. Postictal depression.
2) Fleeting impairments of consciousness, child assumed daydreaming.
3) Shock-like jerks, can result in sudden falls. Common in children with structural neurological disorder.
What are some types of focal epileptic seizures?
- Simple partial: twitching/jerking one side of face/arm/leg. Consciousness retained. Jacksonian march. Sometimes progresses to full tonic-clonic.
- Complex partial: altered consciousness a/w strange sensations, hallucinations. Commonly chewing, sucking + swallowing movements. Postictal phase.
What is a Jacksonian march?
When jerking starts in one part of the body and spreads.
What are infantile spasms? (West Syndrome!)
Form of myoclonic epilepsy but considered separately as have particularly poor prognosis.
Presentation of Infantile spasms?
- Age of onset 3-8months
- Typical flexion spasms (knife jerk/ ‘salam’ spasms) lasting few secs, in clusters up to 30mins.
- Usually occur on waking from sleep.
- ?Hx perinatal complications (meningitis/asphyxia)
- Following onset= developmental regression.
- EEG characteristic ‘hyperrhythmic’ (chaotic) pattern.
What genetic association is a/w infantile spasms?
SCN1A mutation!!!
EEG is indicated when epilepsy is suspected. What are the characteristic patterns for:
- Simple absence (petit mal) seizures
- Infantile spasms (West syndrome)
- Three per second spike + wave discharge, B/L synchronous.
2. Hypsarrhythmia, + chaotic background of slow-wave activity.
Management of Infantile Spasms?
- Vigabatrin (1st line)
- ACTH hormone, or prednisolone.
What is the 1st line management of all generalised seizures in children?
Valproate
- Except in females of child bearing age!!
- Also, if the epilepsy is established + tonic-clonic seizures ONLY then chose : Lamotrigine!
What is not recommended in all generalised seizures apart from tonic-clonic?
Carbamazepine, gabapentin or phenytoin.
these are NOT recommended in absense/ myoclonic/ tonic/atonic seizures
What is generally second line in all generalised seizures?
Lamotrigine
What is 1st line mx in focal seizures?
Lamotrigine!
Also can use: Carbamazepine, Valproate.
What are the key s/e of Valproate?
- Wt gain, hair loss
- Rare idiosyncratic liver failure (need to monitor LFTs)
- High teratogenic potential (Pregnancy Prevention Program if no alternative)
What are the s/e of Carbamazepine/Oxcarbazepine?
- Rash, neutropenia, hyponatraemia, ataxia.
- P450 INDUCER!
S/e of Topiramate?
Nasty!
-Drowsiness, withdrawal, wt loss.
S/e of Vigabatrin?
- Restriction of visual fields
- Sedation
Mx of Acute Seizures in children?
- Recovery position. Rectal Diazepam, or Buccal midazolam
- If seizure continues >25mins : phenytoin
- If seizure continues >45mins : anaesthesia
Tension headaches:
a) Character
b) Timing
c) Mx
a) Constricting, band like. Usually develop towards later childhood.
b) End of day. Stress as home/school.
c) Reassurance, paracetamol. Minimise attention to them!
Migraine without aura (90% of migraines):
a) Character
b) Timing
c) Associated features
a) Throbbing/pulsatile over temporal/frontal areas. Usually develop late childhood/early teens.
b) Episodic (1-72hrs)
c) GI disturbances, photo/phonophobia. Aggrivated by physical activity!
Management of migraine without aura?
- Rest, simple anaesthesia.
- Diet control
- If frequent: prophylactic propranolol/pizotifen.
- In teens: sumatriptan.
What is the character of a migraine with aura?
Headache is preceded by an aura (visual/sensory/motor).
Aura:
-Negative phenomena: hemianopia, scotoma (small areas visual loss)
-Positive phenomena: fortification spectra (zig-zag line)
Mx of migraine with aura?
Sleep often relieves.
There is a presence of premonitory sx: tiredness, poor conc., autonomic feats
Red Flags in a child with headache
- Acute onset/ severe pain
- Fever
- Intensifies lying down
- A/w vomiting
- Regression of developmental skills
- Consistently U/L pain
- Cranial bruit
- HTN or papilloedema
What are some characteristic features of ^ICP or space occupying lesions?
- Worse lying, morning vomiting (without nausea)
- Night time waking
- Change in mood/ personality/ educational performance.
Head injury in a child: what advice can be given in a pre-hospital setting?
Advise that child must go to hospital if:
- Any LoC/ seizure
- High energy head injury
- Any focal neurological deficit since the injury
- Any amnesia
- Any vomiting
- Safeguarding concerns
What are the signs for a basal skull fracture?
- Panda eyes (peri-orbital bruising)
- Battle’s sign (mastoid bruising)
- CSF leaking from nose/ear
What is the difference between non-communicating + communicating hydrocephalus?
Non-communicating (obstructive)= obstruction within the ventricular system/ aquaduct.
Communicating= obstruction at the arachnoid villi (site of CSF absorption)
Causes of non-communicating hydrocephalus?
- Congenital: aquaduct stenosis, Dandy-Walker malformation (outflow atresia), Chiari malformation.
- Posterior fossa neoplasm
- Intraventricular haemorrhage (in prem)
Causes of communicating hydrocephalus? (failure to reabsorb CSF)
- Subarachnoid haemorrhage
- Meningitis (pneumococcal, TB)
What is hydrocephalus commonly a/w?
Neural tube anomalies (occurs in 80% spina bifida)
Presentation of hydrocephalus in infants?
obviously this varies with age + rate in ^ICP
Common: irritability, lethargy, poor appetite, vomiting.
- Accelerated head growth (anterior fontanelle open + bulging, sutures separated)
- Broad forehead, eyes deviated down (‘setting sun’ sign)
- Spasticity, clonus + brisk deep tendon reflexes
Management of hydrocephalus?
- Cranial US/MRI/CT
- Head circumference monitored on centile charts.
- Intracranial shunts (ventriculoperitoneal)
Difference between Plagiocephaly and Craniosynostosis?
Plagiocephaly: asymmetric flattening of one side of skull from positional moulding.
Craniosynostosis: premature fusion of one of more sutures, leading to distortion of head shape.
What is Sturge-Weber Syndrome associated with?
-Haemangiomatous facial lesion (port-wine stain), in the distribution of the trigeminal nerve (a/w similar lesion intracranially). Always involves the ophthmalmic division.
Presentation of Sturge-Weber syndrome?
Most severe: epilepsy, learning disabilities + hemiplegia.
Less severe: deterioration unusual after 5yrs, may still be seizures/learning difficulties.
-^risk glaucoma
What medication can be used for Gilles de la Tourette’s syndrome? (chronic tic disorder)
-Clonidine or resperidone.
What is Duchenne muscular dystrophy?
- Progressive disorder resulting in death in early 20s.
- X-linked recessive, Dystrophic gene defect..
- It is the commonest hereditary neuromuscular disease.
Presentation of Muscular Dystrophy?
- Baby boy normal at birth, delayed walking.
- 4-6yrs: frequent falls, lordotic waddling gait.
- Enlarged, weak calves.
- GOWER’s SIGN (characteristic)
- Creatine kinase level elevated x10
What may a rigid flat foot indicate in older children?
A RIGID flat foot is pathological:
- Tendo-Achilles contracture (ankle)
- Tarsal coalition (lack of segmentation between 1/more bones of foot)
- Juvenile idiopathic arthritis
How does Septic Arthritis present in children? (most common <2yrs)
- Joint usually hot, swollen + acutely tender
- +/- fever
- Limited + painful movement of affected joint
- Joint effusion in peripheral joints
- Initial presentation may be a limp
Mx of septic arthritis?
- IV flucloxacillin
- Joint should be splinted in acute stage, then mobilised to prevent permanent deformity
What is ‘transient synovitis’? (a common DDx of septic arthritis)
Transient synovitis= ‘irritable hip’, the commonest cause of acute hip pain in children.
2-12yrs, following/accompanying a viral infection.
In a few, TS precedes the development of Perthes disease.
What does Reactive Arthritis (commonest form of arthritis in children) usually follow?
Extra-articular infections:
- Children: enteric bacteria (shigella, salmonella, campylobacter)
- Adolescents: viral (chlamydia, gonococcus), mycoplasma + borrelia burgdorferi (Lyme disease).
- Rheum. fever + post-strep in developing countries.
What is the presentation of reactive arthritis/irritable hip?
- Low grade fever
- Transient joint swelling (<6wks), usually ankles/knees
- Acute-phase reactants normal/mildly^, XRays normal
- Generally transient + good outcome (NSAIDs only)
How does Juvenile Idiopathic Arthritis present?
- Gelling (stiffness after rest)
- Morning joint stiffness + pain
- Initially min. joint swelling, then subsequent swelling + inflammation.
- Chronic: proliferation of synovium + swelling of periarticular soft tissues.
What are the long term features of Juvenile Idiopathic Arthritis?
Bone expansion from overgrowth.
Knee: leg lengthening + valgus deformity.
Hands: digit length discrepancy.
(types of JIA)
Characteristics of systemic JIA (Still’s disease)?
- Rarest form
- Articular: large + small joints affected
- Extra-articular: remitting fever, macular rash, hepatosplenomegaly, lymphadenopathy, wt loss.
- Lab results: anaemia, ^neutrophils+platelets.
(types of JIA)
Characteristics of Polyarticular JIA?
- Articular: large+small joints affected symmetrically, morning stiffness.
- Extra-articular: chronic anterior uveitis (5%)
(types of JIA)
Characteristics of Pauciarticular/Oligoarticular JIA?
- Commonest form of JRA
- Articular: <5joints, usually large
- Extra-articular: asymmetrical growth, chronic anterior uveitis (20%! NEED REGULAR OPTHALM!)
What do Rhesus factor/ antinuclear antibodies say in: a) Systemic b) Polyarticular c) Pauci/Oligoarticular (JRA)
a) Negative
b) RhF-ve, ANA+ve
c) RhF-ve, ANA+ve
What are systemic corticosteroids (IV methylprednisolone) used to manage?
-Severe polyarthritis as induction agent.
-Systemic arthritis (life-saving)
Avoided if poss to minimise risk of growth suppression + osteoporosis.
What is the cause of a Limping Child age 3-5yrs?
Hip effusion- usually caused by transient synovitis or infection.
MUST differentiate from septic arthritis!! (difficult on USS, usually need XR)
What is Perthes disease?
Limping, 5-8yrs, M:F 5:1
Idiopathic necrosis of hip (due to interruption in blood supply, followed by revascularisation + reosification over 18-36months).
13% bilateral
What are the Xray findings of a child with Perthes disease?
Fragmented flattened femoral head, subsequently becomes fragmented + irregular.
Mx of Perthes disease?
- If identified early: bed rest + traction.
- Severe/late: maintain hip in abduction w/plasters or calipers (femoral head covered by acetabulum to act as mould for re-ossifying epiphysis)
What is a Slipped Capital Femoral Epiphysis?
Idiopathic fracture through proximal femoral growth plate- results in displacement of epiphysis of femoral head postero-inferiorly.
Limping teenager 12-15yrs, often obese, 1/3 B/L.
What is Slipped Capital Femoral Epiphysis associated with?
Metabolic endocrine abnormalities (hypothyroidism + hypogonadism)
Presentation of Slipped Capital Femoral Epiphysis?
Limp w/hip pain may be referred to knee. Onset acute (post-trauma) or insidious. Examination: restricted abduction + internal rotation of hip.
Recommended isolation for:
a) Measles
b) Mumps
c) Rubella
a) Measles: from onset of catarrhal stage to day 5 of rash.
b) Mumps: until swelling subsides
c) Rubella: none except non-immune women in 1st trimester.
What is the recommended isolation for Chicken Pox?
Until all lesions are crusted over.
What is involved in the ‘5 in 1’ vaccine?
- Diptheria
- Tetanus
- Pertussis
- H influenzae type b (Hib)
- Polio
When should MMR vaccine NOT be given?
- Non-HIV related immunodeficiency.
- Allergy to neomycin/kanamycin.
- Hx of anaphylaxis to egg should be given under supervision.
Much of the damage caused by meningeal infection results from the host response. What are 3 pathophysiologies of this?
- Release of inflam. mediators + activated leukocytes –> endothelial damage –> cerebral oedema, ^ICP + decreased cerebral bloodflow.
- Inflammatory response –> vasculopathy –> cerebral cortical infarction.
- Fibrin deposits may block resorption of CSF by arachnoid villi –> hydrocephalus.
What are the main organisms causing meningitis in:
a) Neonates - 3months
b) 1month - 6yrs
c) >6yrs
a) Group B strep, E. coli, Listeria monocytogenes.
b) Neisseria meningitidis, Strep pneumoniae, Haemophillus influenzae.
c) Neisseria meningitidis, Strep pneumoniae,
What is the difference between where the organism effects in Bacterial + Viral meningitis?
Bacterial: usually confined to meninges.
Virus: may invade underlying brain, causing meningoencephalitis.
What types of virus cause meningitis in infants/children?
- Mumps virus
- Coxsackie & echo viruses (enterovirus)
- Herpes simplex
- Adenovirus
- Poliomyelitis (developing countries)
General presentation of Meningitis in infants/children?
- Papilloedema rare in children
- Bulging fontanelle late sign in infants.
- Neck stiffness not always present.
- Opisthotonus (arching of back)
Viral presentation of Meningitis?
- Preceded by pharyngitis/GI upset
- Then fever, headache + neck stiffness (not reliable sign)
- Classical head retraction a late feature (contrasting to adults)
Bacterial presentation of Meningitis?
- Early feature = reduction in consciousness level
- High pitched cry
- Convulsions in infants
- In meningococcal disease: petechial haemorrhages > purpuric rash.
What do you assume if a febrile child presents with Purpura?
Assume to be due to meningococcal sepsis, even if child doesn’t appear unduly ill at time.
-Give IV BENZYLPENICILLIN + transfer to hospital.
Mx of viral/bacterial meningitis?
Viral: usually self-limiting.
Bacterial: IV cefotaxime/ ceftriaxone 10-14days (3rd gen cephalosporins)
-Also dexamethasone beyone neonatal period!
What do household contacts of Neisseria meningitidis receive?
Prophylactic Rifampicin (along w/infected child after Abx). Haemophilus influenzae injection.
Contraindications to a Lumbar puncture in suspected meningitis? (to examine CSF- cloudy in bacterial)
- Signs of raised ICP (papilloedema, low HR, high BP)
- Focal neuro signs
- Cardiorespiratory instability
- Coagulopathy
- Thrombocytopenia
- Local infection at site of LP
Describe the following signs that are associated with neck stiffness:
a) Brudzinski sign
b) Kernig sign
a) Brudzinski sign: flexion of neck w/child supine causes flexion of knees + hips.
b) Kernig sign: when child lying supine w/hips + knees flexed, there is back pain on extension of knee.
What are the key differences in the CSF between bacterial + viral meningitis?
Colour: cloudy in bacterial, clear in viral.
Protein: ^^in bacterial, ^/-in viral.
Glucose: decreased in bacterial, normal/decreased in viral.
What is the commonest long-term complication in VIRAL meningitis?
Sensorineural hearing impairment.
What are some complications of BACTERIAL meningitis?
- Hydrocephalus (communicating or non-communicating, when blocked ventricular outlet by fibrin)
- Subdural effusion (a/w haemophilus influenzae + pneumococcal meningitis)
- Hearing loss
- Local vasculitis (may lead to CNS palsies)
- Cerebral infaction (may lead to focal seizures + epilepsy)
- Acute adrenal failure
What are some general causes of purpura/easy bruising?
- Thrombocytopenia (^platelet destruction/consumption, impaired platelet production)
- Platelet count normal: vascular disorders (CT disorders, meningococcal infection, Henoch-Schonlein purpura, SLE.
Presentation of Immune Thrombocytopenic Purpura?
- Acute onset (1-2wks after viral infection)
- Reduced platelet count, Increased megakaryocytes
- Petechiae + superficial bruising
- Mucosal bleeding
- Child appears clinically well
- Intracranial haemorrhage in 1%
Mx of Immune Thrombocytopenic Purpura?
- If mild: none (usually remit spont. 6-8wks)
- Platelet transfusion if v low count
- Supportive mx if becomes chronic ITP (20%)
What is Autoimmune thrombocytopenic purpura caused by?
Maternal IgG antibodies crossing placenta + damaging foetal platelets –> foetus becomes thrombocytopenic.
What is the pathophysiology of Disseminated Intravascular Coagulation (DIC)?
-Coagulation pathway activation, leading to diffuse fibrin deposition in the microvasculature + consumption of coagulation factors + platelets.
What is the commonest cause of activation of coagulation in DIC?
Severe sepsis + shock due to circulatory collapse (e.g. meningococcal septicaemia)
What investigation results should point to DIC?
When the following coexist:
- Thrombocytopenia
- Prolonged PTT/ATPP
- Low fibrinogen, raised fibrinogen degradation product
- Raised D-dimer
- Microangiopathic haemolytic anaemia
What is Purpura fulminans associated with?
- Mostly: Meningococcal disease (vasculitis- leads to loss of large areas of skin by necrosis).
- Also rarely occurs after VZV infection.
What is the commonest cause of septicaemia in neonates?
Group B strep, or Gram -ve organisms (from birth canal)
What is the physiology of septicaemia?
Host releases inflammatory cytokines + activates endothelial cells.
(commonest cause in childhood is meningococcal infection, which may/not be accompanied by meningitis)
What is the dermatological presentation of chick pox? (varicella)
Rash first appears on trunk/face –> progresses to peripheries.
Papules to Vesicles to Pustules + then crusts over.
What is the mx for adolescents/adules with a primary infection of chicken pox?
Oral valaciclovir
What is Parvovirus B19 (‘slapped-cheek syndrome’)?
Causes erythema infectiousum or Fifth disease.
Erythema infectiousum:
Viraemic phase (fever, malaise, headache), then characteristic facial rash, then macpap ‘lace’ like rash on trunk/limbs.
What is the most serious consequence of Parvovirus?
Aplastic crisis- occurs in children with chronic haemolytic anaemias (sick cell/ thalassaemia), and immunodeficiency (malignancy).
What is the characteristic presentation of Conjunctivitis from:
a) Gonococcal infection
b) Chlamydia infection?
a) First 48hrs of life: purulent discharge w/conjunctival infection + swollen eyelids. Need to culture + IV cephalosporin.
b) 1-2wks of life: purulent discharge + swollen eyelids. PO erythromycin 2wks.
Food allergies can be IgE-mediated and non-IgE mediated, what is the difference?
IgE-mediated: hx of allergic sx from urticaria to anaphylaxis (10mins after ingestion).
Non-IgE mediated: involves GI tract (D+V, abdo pain, failure to thrive), colic/eczema may be present (usually hours after ingestion).
Diagnosis of food allergies?
IgE- mediated: skin-prick tests + measure specific IgE Abs in blood (RAST test)
Non-IgE mediated: clinical Hx.
Gold standard (for both): exclusion of relevant food under a dietician’s supervision.
